Combination of S-adenosylmethionine and mouthguard for oral self-mutilation in Lesch-Nyhan syndrome. Issue 4 (July 2021)

• Record Type: Journal Article
• Authors: Narai, Takashi; Harada, Yusei; Fujii, Nobuyuki; Tamura, Takayuki; Doi, Rieko; Kodani, Isamu
• Is Part Of: Journal of oral and maxillofacial surgery, medicine, and pathology. Volume 33:Issue 4(2021)
• Page Start: 478
• Page End: 481
• Publication Date: 2021-07
• Subjects: Lesch-Nyhan syndrome -- Oral self-mutilation -- S-adenosylmethionine -- Mouthguard; Mouth -- Surgery -- Periodicals; Face -- Surgery -- Periodicals; Maxilla -- Surgery -- Periodicals; Oral medicine -- Periodicals; Mouth -- Diseases -- Pathogenesis -- Periodicals; Surgery, Oral -- Periodicals; Oral Medicine -- Periodicals; Pathology, Oral -- Periodicals; Face -- Surgery; Maxilla -- Surgery; Mouth -- Diseases -- Pathogenesis; Mouth -- Surgery; Oral medicine; Electronic journals -- Sciences; Electronic journals -- Medicine; Periodicals; 617.522059
• DOI: 10.1016/j.ajoms.2021.02.010
• Languages: English
• ISSNs: 2212-5566

Abstract

Abstract: Lesch-Nyhan syndrome (LNS) is characterized by frequent oral self-mutilation (OSM). Although various treatments have been suggested, our limited knowledge regarding the exact pathogenic mechanism of self-mutilation in LNS makes it difficult to manage self-injurious behavior and there is a need for novel treatments. Herein, we describe the case of a 11-year-old boy with LNS and OSM who was conservatively treated using S-adenosylmethionine (SAM) and a mouthguard. This resulted in significant improvement in OSM, and the patient remains stable at 16 years old. This case demonstrates that combination treatment with SAM and a mouthguard should be considered for the management of OSM in LNS, as it can maintain the functionality and health of the oral cavity and improve a patient's quality of life.